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Abstract
Aim: To determine the clinical presentation, biochemical ndings, complications, clinical diagnosis and management of patients with acute fatty liver of pregnancy (AFLP). The inuence of the mode of delivery on
maternalperinatal mortality is analyzed and two methods of diagnostic imaging are compared.
Material & Methods: Eleven cases of AFLP identied at the West China Second Hospital of Sichuan University from January 2003 to June 2008 were studied. To provide additional evidence, 342 cases of AFLP were
reviewed retrospectively from original articles researched in the China National Knowledge Infrastructure
between the same period.
Results: All cases presented with a prodrome of nausea, vomiting, malaise and jaundice. Raised transaminases
and serum bilirubin were found in all patients (100%), hypoglycemia was found in two patients (18.2%) and
hypoproteinemia was found in ve patients (45.5%). All cases were conrmed by diagnostic criteria and
clinical assessment. Diagnostic imaging helped to conrm the diagnoses. Ultrasound was used to diagnose
nine patients (81.8%), and two (18.2%) patients were diagnosed by computerized tomography. Except for one
patient whose labor was induced with oxytocin because of fetal death before admission, all other pregnancies
were terminated by cesarean section within 24 h of denitive diagnosis. All patients and neonates survived
delivery. One (9.1%) maternal death and one (7.2%) fetal death occurred.
Conclusions: Early diagnosis, termination of pregnancy by Cesarean section as soon as possible and comprehensive therapy are crucial for improving the prognoses of both mother and newborn.
Key words: acute fatty liver of pregnancy, diagnosis, treatment.
Introduction
Acute fatty liver of pregnancy (AFLP) is a rare, but
potentially fatal, complication of the third trimester of
pregnancy. Its exact pathogenesis is unknown despite
the accumulation of a signicant amount of data
about the disease. The incidence of AFLP is 1/7000 to
1/16000.1 Although mortality due to AFLP has
declined in recent years, it is important to further
lower the mortality rate by early diagnosis and
treatment of this disease. Some cases of AFLP
have been analyzed retrospectively for improvement
of comprehensive knowledge. Many studies have
751
Q. Wei et al.
Methods
From January 2003 to June 2008, 11 cases of AFLP were
identied at the West China Second Hospital, Sichuan
University. Presenting symptoms, clinical course, laboratory values, maternal complications, clinical diagnosis, management and maternalperinatal outcomes
were studied. The mean maternal age at diagnosis
was 27 years (range 2134 years). The mean gestational
age at AFLP diagnosis was 36.4 weeks (range
2838 weeks). All patients were primigravidae. There
were nine single pregnancies and two twin pregnancies. Fetal sex included nine males and four females.
The diagnosis of AFLP was made on the basis of
clinical and laboratory criteria as follows: (i) patients
with symptoms of anorexia, fatigue, nausea, vomiting,
jaundice and abnormal liver function in the third trimester of pregnancy; (ii) characteristic laboratory
examination; (iii) ultrasound imaging showing fatty
liver; (iv) viral hepatitis, pharmaceutic hepatitis, toxic
hepatic and other hepar diseases complicating pregnancy were excluded by laboratory examination; and
(v) liver biopsy in accordance with pathologic changes.
All cases conformed to the diagnostic criteria mentioned above, except for liver biopsy. Because of their
severe conditions, prolonged prothrombin times,
reduced platelet counts, and/or the patients refusal,
two patients did not receive liver biopsy.
Furthermore, as additional research, 342 cases of
AFLP were reviewed retrospectively from original
articles searched in the China National Knowledge
Infrastructure between January 2003 and June 2008. In
our data, all patients conformed to the diagnostic criteria mentioned above. In the present study, the data was
mainly used to analyze the clinical diagnosis, the inuence of the mode of delivery on maternalperinatal
outcomes, and to compare the two methods of diagnostic imaging used in the examination of patients
diagnosed with AFLP. The contents of this research will
be embodied in the discussion.
Results
Of the 11 patients, all cases presented with anorexia,
fatigue, nausea, vomiting, jaundice and abnormal liver
function in the third trimester of pregnancy. Five cases
had disseminated intravascular coagulation (DIC), four
had renal insufciency, two had hypertensive disorder
complicating pregnancy, three had edema and ascites,
three had hepatic encephalopathy, ve had hypoproteinemia, one had upper gastrointestinal hemorrhage,
752
Percentage
(%)
5
4
3
5
2
1
1
2
1
45.5
36.4
27.3
45.5
18.2
9.1
9.1
18.2
9.1
9
2
9
81.8
100
100
10
1
90.9
9.1
1
0
0
1
9.1
0
0
7.7
Range
Average
Reference
value
67425
46578
78445
60370
1832.5
6.941.0
68659.6
17.935.6
40126
8.621.8
1.910.8
134.6
278.9
156.2
102.5
23.6
15.7
217.5
28.7
58.9
14.6
5.4
050
050
2.024.0
07.0
3650
1.787.14
30.697.3
8.514.5
100450
4.010.0
<11.1
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Q. Wei et al.
Discussion
AFLP is a late-gestational complication, often occurring
at 2840 weeks (mean 36 weeks). It is reported that
being primigravida, having had multiple pregnancies,
carrying a male fetus, and experiencing preeclampsia
are the high-risk factors for AFLP. Despite of extensive
investigations for the genetic origins of pre-eclampsia
and related diseases, the causes of hemolysis, elevated
liver enzymes, and low platelet count syndrome
(HELLP syndrome) and AFLP have not been claried
yet.24 Pre-eclampsia occurs in approximately 50% of
AFLP cases, and 15% of cases are associated with multiple pregnancies.5,6 The morbidity ratio for AFLP
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Percentage
(%)
145
140
112
198
232
111
86
108
82
41.1
39.7
31.7
56.1
65.7
31.4
24.4
30.6
23.2
189
28
55
79.7
85.3
100
228
104
68.7
31.3
37
50
32
36
16.2
48.1
10.6
26.5
between male and female fetuses is 3 : 1. Our data conforms to the situation mentioned above. Because the
initial symptoms of AFLP are atypical and could be
neglected, and because this disease develops rapidly
and causes multi-system dysfunction in a very short
time, it is important to be especially vigilant for its
development. Summarizing the data from 353 cases,
AFLP should be highly suspected when the following
clinical conditions occur: (i) gastrointestinal symptoms,
which include nausea, vomiting, fatigue, and vague
abdominal pain, appearing in late pregnancy without
obvious reason; (ii) jaundice occurring after gastrointestinal symptoms and promptly becoming worse; (iii)
liver dysfunction occurring in late pregnancy, when
other hepatic diseases have been excluded; (iv) laboratory examination showing obvious leukocyte elevation,
decreased red blood cells and platelets, elevated total
bilirubin, especially direct bilirubin, and an increase in
hepatic aminotransferase levels (ALT, AST) from mild
to moderate (the variable range was reported from
normal to 1000 U/L and seldom over 500 U/l. Most
research reports the mean to be approximately 200 U/
l.); (v) hypertensive disorder complicating pregnancy
associated with hypoglycemia, hypobrinogenemia,
and increased prothrombin time; (vi) maternal coagulation dysfunction complicated with fetal distress in
late pregnancy without denitive reason; and (vii)
755
Q. Wei et al.
References
1. Chng CL, Morgan M, Hainsworth I, Kingham JG. Prospective study of liver dysfunction in pregnancy in Southwest
Wales. Gut 2002; 51: 876880.
2. Bienertov-Vasku J, Dostlov Z, Kankov K, Bienert P, Vasku
A, Unzeitig V. Is there any link between severe pre-eclampsia
and dened polymorphisms in leptin and adiponectin genes?
J Obstet Gynaecol Res 2008; 34: 858864.
3. Omar SZ, Qvist R, Khaing SL, Muniandy S, Bhalla S. Thrombophilic mutations in pre-eclampsia and pregnancy-induced
hypertension. J Obstet Gynaecol Res 2008; 34: 174178.
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